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  • Title: Alpha-1-antitrypsin deficiency panniculitis.
    Author: Valverde R, Rosales B, Ortiz-de Frutos FJ, Rodríguez-Peralto JL, Ortiz-Romero PL.
    Journal: Dermatol Clin; 2008 Oct; 26(4):447-51, vi. PubMed ID: 18793976.
    Abstract:
    Alpha-1-antitrypsin deficiency is a congenital error of metabolism linked to pulmonary (emphysema) and liver (cirrhosis) disease. Since 1972, panniculitis has been associated with this deficiency, initially related to Weber-Christian syndrome and finally as a differentiated entity. Clinical manifestations typically consist of wide nodular lesions on the trunk and proximal extremities that evolve to ulceration and drainage. Histopathologically it presents as a mixed septal-lobular panniculitis pattern with some typical findings referred. Differential diagnosis from other types of panniculitis and neutrophilic dermatosis must be established. Different treatments, including tetracyclines, dapsone, and alpha-1-antitrypsin repositioning, have shown variable efficacy in controlling this disease.
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